IKZF1 (Ikaros family zinc finger 1)
Written | 2000-08 | Jean-Loup Huret |
Genetics, Dept Medical Information, University of Poitiers, CHU Poitiers Hospital, F-86021 Poitiers, France |
(Note : for Links provided by Atlas : click)
1. Identity
Alias_names | General Information |
ZNFN1A1 | |
Alias_symbol (synonym) | hIk-1 |
LyF-1 | |
Hs.54452 | |
IKAROS | |
PPP1R92 | |
Other alias | IK1 |
LYF1 | |
ZNFN1A1 (zinc finger protein, subfamily 1A, 1) | |
HGNC (Hugo) | IKZF1 |
LocusID (NCBI) | 10320 |
Atlas_Id | 258 |
Location | 7p12.2 [Link to chromosome band 7p12] |
Location_base_pair | Starts at 50304669 and ends at 50405100 bp from pter ( according to hg19-Feb_2009) [Mapping IKZF1.png] |
Fusion genes (updated 2017) | Data from Atlas, Mitelman, Cosmic Fusion, Fusion Cancer, TCGA fusion databases with official HUGO symbols (see references in chromosomal bands) |
BCL6 (3q27.3) / IKZF1 (7p12.2) | CREB3L2 (7q33) / IKZF1 (7p12.2) | ENTPD1 (10q24.1) / IKZF1 (7p12.2) | |
IKZF1 (7p12.2) / ABCA13 (7p12.3) | IKZF1 (7p12.2) / B2M (15q21.1) | IKZF1 (7p12.2) / BCL6 (3q27.3) | |
IKZF1 (7p12.2) / CDK2 (12q13.2) | IKZF1 (7p12.2) / ETV6 (12p13.2) | IKZF1 (7p12.2) / IKZF1 (7p12.2) | |
IKZF1 (7p12.2) / LRBA (4q31.3) | IKZF1 (7p12.2) / NUTM1 (15q14) | IKZF1 (7p12.2) / POLR2A (17p13.1) | |
IKZF1 (7p12.2) / PRDM16 (1p36.32) | IKZF1 (7p12.2) / SETD5 (3p25.3) | IKZF1 (7p12.2) / TRPV2 (17p11.2) | |
IKZF1 (7p12.2) / ZEB2 (2q22.3) | SETD5 (3p25.3) / IKZF1 (7p12.2) | STIM2 (4p15.2) / IKZF1 (7p12.2) | |
2. DNA/RNA
Transcription | 3629 bp mRNA; coding sequence: 1559 bp; 6 different splicings (--> 6 isoform proteins: ik1-ik6) |
3. Protein
Description | 519 amino acids; 58 kDa; possesses 2 Zinc-finger (C2H2-type) domains: one with 4 ZnF, the second with2 ZnF; DNA binding |
Expression | specificity for hematopoietic organs in the foetus and in the adult as well |
Localisation | nuclear |
Function | transcription regulator; can repress transcription through the recruitment of histone deacetylase complexes; role in conjunction with Aiolos; hemopoietic-specific zinc finger protein regulator of B and T-cell differentiation |
4. Implicated in
Note | |
Entity | t(3;7)(q27;p12) diffuse large B-cell lymphoma (DLCL) --> BCL6 / IKZF1 |
Note | only 2 cases to date |
Hybrid/Mutated Gene | 5' Ikaros - 3' BCL6 fusion transcript; it is supposed that substitution of the promoter of BCL6 may be responsible for BCL6 deregulation |
5. Bibliography
Association of transcriptionally silent genes with Ikaros complexes at centromeric heterochromatin. |
Brown KE, Guest SS, Smale ST, Hahm K, Merkenschlager M, Fisher AG |
Cell. 1997 ; 91 (6) : 845-854. |
PMID 9413993 |
The role of the Ikaros gene in lymphocyte development and homeostasis. |
Georgopoulos K, Winandy S, Avitahl N |
Annual review of immunology. 1997 ; 15 : 155-176. |
PMID 9143685 |
The Ikaros gene, a central regulator of lymphoid differentiation, fuses to the BCL6 gene as a result of t(3;7)(q27;p12) translocation in a patient with diffuse large B-cell lymphoma. |
Hosokawa Y, Maeda Y, Ichinohasama R, Miura I, Taniwaki M, Seto M |
Blood. 2000 ; 95 (8) : 2719-2721. |
PMID 10753856 |
Ikaros interactions with CtBP reveal a repression mechanism that is independent of histone deacetylase activity. |
Koipally J, Georgopoulos K |
The Journal of biological chemistry. 2000 ; 275 (26) : 19594-19602. |
PMID 10766745 |
Defects in hemopoietic stem cell activity in Ikaros mutant mice. |
Nichogiannopoulou A, Trevisan M, Neben S, Friedrich C, Georgopoulos K |
The Journal of experimental medicine. 1999 ; 190 (9) : 1201-1214. |
PMID 10544193 |
Pre-T cell receptor (TCR) and TCR-controlled checkpoints in T cell differentiation are set by Ikaros. |
Winandy S, Wu L, Wang JH, Georgopoulos K |
The Journal of experimental medicine. 1999 ; 190 (8) : 1039-1048. |
PMID 10523602 |
Identification of heterologous translocation partner genes fused to the BCL6 gene in diffuse large B-cell lymphomas: 5'-RACE and LA - PCR analyses of biopsy samples. |
Yoshida S, Kaneita Y, Aoki Y, Seto M, Mori S, Moriyama M |
Oncogene. 1999 ; 18 (56) : 7994-7999. |
PMID 10637510 |
6. Citation
This paper should be referenced as such : |
Huret, JL |
IKZF1 (Ikaros family zinc finger 1) |
Atlas Genet Cytogenet Oncol Haematol. 2000;4(4):179-180. |
Free journal version : [ pdf ] [ DOI ] |
On line version : http://atlasgeneticsoncology.usal.es/classic/Genes/IkarosID258.html |
7. Other Leukemias implicated (Data extracted from papers in the Atlas) [ 12 ]
Other Solid tumors implicated (Data extracted from papers in the Atlas) [ 1 ] |
t(4;7)(q31;p12) IKZF1/LRBA
|
8. External links
REVIEW articles | automatic search in PubMed |
Last year publications | automatic search in PubMed |
© Atlas of Genetics and Cytogenetics in Oncology and Haematology | indexed on : Thu Jan 17 18:58:02 CET 2019 |
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